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Doose Syndrome

What is Doose Syndrome?

Doose syndrome is a seizure disorder characterized by frequent myoclonic and myoclonic-atonic seizures. “Myoclonic seizures” cause jerking or twitching in the arms and legs. “Atonic seizures” cause a loss of muscle control. Atonic seizures are also called “drop” or “astatic” seizures. Doose syndrome is sometimes called “myoclonic-astatic epilepsy.”

CChildren with Doose syndrome may have other types of seizures as well. One example is generalized tonic-clonic seizures. These types of seizures involve stiffening of the body and / or arms and legs. This can be followed by rhythmic jerking.

The disorder is rare. It affects 1-2% of all children with epilepsy. It is more common in boys than girls. Signs and symptoms typically begin between 2 and 6 years of age.

For children with Doose syndrome, seizures are very hard to control completely with typical anti-seizure medications. There is no cure for Doose syndrome. Many children can get at least some seizure relief with a ketogenic or modified Atkins diet. Some children will outgrow Doose syndrome.

Doose Syndrome Causes

No one knows what causes Doose syndrome. One or more gene changes are responsible for the disorder. The only known risk factor is a family history of seizures. But about two-thirds of children with Doose syndrome do not have a known family history of epilepsy

Doose Syndrome Symptoms

All children with Doose syndrome have many myoclonic and myoclonic-atonic seizures. When myoclonic-atonic seizures happen, the child suddenly goes limp. They may fall to the ground if they are standing. In addition, children with Doose syndrome may have other types of seizures , including:

  • Tonic-clonic seizures
  • Absence seizures—The child may appear to be daydreaming or zoning out.
  • Tonic seizures——These types of seizures involve stiffening of the body and/or arms and legs. Tonic seizures are typically shorter than tonic-clonic seizures.
  • Atonic seizures
  • Non-convulsive status epilepticus—These are subtle seizures lasting more than five minutes. During these types of seizures, the child may or may not be aware they are happening.
  • Other types of status epilepticus seizures.

Children with Doose syndrome can have seizures that occur alone or in clusters. This may happen hundreds of times a day. Seizures tend to occur more often in the morning. Children with Doose syndrome need constant supervision. A protective helmet can reduce the risk of head injury.

Frequent seizures can sometimes lead to learning disabilities and developmental delays. These problems can range from mild to significant in severity.

Doose Syndrome Diagnosis

Doctors diagnose Doose syndrome based on many factors. These include:

  • The child’s seizure history (seizures typically begin between 2 and 6 years of age).
  • A physical and neurologic examination.
  • An electroencephalogram (EEG). This is a test that detects electrical activity in the brain.

Doose Syndrome Treatment

The goal of treatment is to help children with Doose syndrome have the best quality of life possible. That happens by trying to reduce the number of seizures. Treatment for Doose syndrome may include:

  • Dietary therapy with a ketogenic or modified Atkins diet.
  • Medications that target generalized seizures, including ethosuximide, valproic acid, lamotrigine, levetiracetam, topiramate, zonisamide, clobazam and felbamate.

Dietary therapy is often the first-line treatment for Doose syndrome. Typical anti-seizure medications (including those listed above) rarely control all seizure types seen in Doose syndrome. But sometimes, a combination of anti-seizure drugs can provide partial relief of at least one or more seizure types.

Careful drug selection is critical. Some anti-seizure medications (for example, carbamazepine) may make the condition worse.

Doose syndrome is not the kind of epilepsy that can be treated by surgically removing brain tissue that is causing seizures. The seizures associated with Doose syndrome are generalized. This means they are caused by abnormal electrical activity on both sides of the brain rather than from a single location in the brain.

A holistic care plan should also include treating related problems, like learning disabilities,—with specific therapies learning plans.

Doose Syndrome Prognosis

About two-thirds of children with Doose syndrome will stop having seizures. They will no longer need any medical treatment. Other children experience seizure control through one or more types of treatment. When seizures stop happening, the child’s development often improves. Other children continue to have many seizures a day. They may also have mild to severe developmental and learning delays.

Factors such as the severity of a child’s condition, how often seizures occur and the type of seizures a child has do not help doctors predict a child’s long-term outcome.

Last Updated 07/2022

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